Monday, December 25, 2017

We got the gift of some great news this week, after spending nearly 4 hours at our quarterly clinic visit, enhanced by pulmonary function tests, chest x-ray, and a blood draw. Thanks to all the various poking and prodding, we now know that Lemon is 1.5cm taller than he was 3 months ago, that his chest x-ray looked clear, and that he did really well on the pulmonary function test/ His iron levels are good, and most amazingly, his liver enzymes are normal. For the first time since we've been measuring them. I could hardly believe it. We've had a wonderful holiday visit with Grandma and Grandpa, and the boys have gotten so many nice things from so many generous people. But, nothing for me will top seeing so many normal test results come in at once. I hope all of you had a wonderful time celebrating whatever you celebrate, and that the coming year brings you abundant happiness and good health. See you in 2018!

Monday, December 18, 2017

On the week of a clinic visit, I always try to put together in my mind what has happened in the 3 months since we were last seen, and try to figure out what parts of our plan need to be optimized going forward. I needn't remind most of you that the past 3 months have been a fairly unrelenting series of respiratory infections of various types and degrees of severity. Still, I feel like in spite of all that, our respiratory plan is working. We have fought off all but two of those things without any antibiotics at all, and the two times that we felt like we did need to use an antibiotic, we had Cayston on hand, deployed it, and beat things back to baseline. We've just finished a week on Cayston now and Lemon is back to respiratory baseline already, so I'm pretty sure we can stop after 2 weeks, or maybe after 10 days if our care team agrees.

If I have learned anything over 4.5 years of being a CF mama, it's that if it's not respiratory, it's nutrition. That is indeed the case now--Lemon is currently lighter than he was in June, and also taller--I'll be curious to see by how much, but I can see his ankles sticking out of the new pants I bought him for school this fall. When weight goes down and height goes up, BMI goes in the toilet, and I can see it written all over his body, every rib, every vertebra, every muscle in his back clearly visible. He's not unhealthily skinny (yet, I think), but he is definitely very thin. Thin would be OK if I didn't have this suspicion that our reserves would be called upon many more time this winter, and we don't have any right now.

So, what am I hoping to get out of this clinic visit? Well, we will get a lot of data. It's our big annual recheck of everything, which means not just the regular two-hour song and dance, but also pulmonary function tests, chest x-ray, and a blood draw. I am curious to see how Lemon's lungs are doing in light of all they've been through so far this winter, although I doubt either the PFTs or the x-rays will tell us much, because Lemon isn't reliable enough at doing the PFTs yet for the data to be really valid, and an x-ray is not exactly a highly sensitive measure of how lungs are doing. I will also be very curious to see how his liver numbers are after 3 months on ursodiol--hopefully it's working and his numbers will be within the normal range, or at least closer to it. We'll also get a vitamin D level to see if he's taking enough vitamins, and some iron measures to see if the amount we're supplementing is the right amount

.

I'm also hoping to continue to get some new ideas from our nutritionist on how to add calories to Lemon's formula so we can get some weight back on him. I've given up all short-term hope of Lemon actually eating enough calories to have any kind of impact on his weight. With his BMI continuing to drop, I basically just want to ram as many calories as is feasible through the tube to get some weight on him over the next couple of weeks, when he'll be home from school and away from the 18 pathogen-carrying monsters (ahem, I mean his friends) in his class. We're continuing with food school for now. I had thought about pulling the plug after today's session, because Lemon had really started to resist going. I mentioned that to his therapist, and she said that she wasn't at all surprised, that she's been pushing his abilities by offering increasingly challenging foods. And Lemon being Lemon, he doesn't want to do things he's not good at so he'd rather just not go. She still thinks he's making progress, so we'll keep at it for a while longer and see where we can get.

Assuming we survive the clinic visit, we're all looking forward to the upcoming holidays. This is the first year Lemon has been old enough to really anticipate the holidays (well, mainly all his presents). Lime still doesn't quite get the idea of presents yet, but he loves all the lights and decorations. And he still can't quite say the "k" sound (usually substituting "t"), so his pronunciation of "Christmas" cracks me up every time. Don't worry, I'm still a committed Scrooge at heart, but I do appreciate seeing the magic of the holiday season through the kids eyes.

Monday, December 11, 2017

Thus far, our fall and winter has been kind of like the experience of being on a small boat at sea. You get buffeted by a wave, then the wave disappears. Is it calm, or is it a trough between waves? Trough. Another wave comes and goes. Calm? Nope, another trough. Another wave, calm again. Or so it seems. Trough after all. Having played a few rounds, I'm pretty confident that the answer is pretty much always trough. Some troughs may be wider than others, just to kind of mix it up, but they are all troughs in the end.

Some may say that I jinxed our last pseudo-calm by noting how calm it was. Not so. In fact, at the time that I wrote last week's post, Lime was already beginning to cough and sneeze as a result of cold #307 that was about to make its way through our family. But, I wanted to dwell for a little while longer on that flat stretch of water. As it turns out, #307 was quite a doozy. Lime was perky enough on Tuesday morning, so I brought him to school as usual. When I picked him up, though, he was in a sorry state of affairs and his teacher said to me, "Maybe not tomorrow." So, I arranged for our former nanny to come and watch him on Wednesday morning. Of course, by Tuesday night, Lemon was coughing too, and since I had childcare arranged at home anyhow I let him stay home, too. I might point out this is the first day of school he has missed this year--quite an achievement on his part.

On Thursday, both guys were feeling pretty lousy so I kept them both home again, but on Friday they both seemed quite improved so back to school they went. Lime continued to recover, whereas Lemon's recovery sort of stalled, and he continued to look really pale. I mean even paler than usual. In spite of his color and the cough he seemed sprightly enough until yesterday morning, when the cough took a marked turn for the worse--with Lime's cough almost completely gone, Lemon's was suddenly big, wet, and productive. I did not like the sound of that at all, and Papa Bear and I agreed immediately that it was time to start Cayston again. That was definitely the right call, because 24 hours in to the course of Cayston, Lemon's cough has nearly dried up and he seems to be feeling quite a bit better. Hopefully this means we can get away with just doing 2 weeks this time.

Maybe we'll get lucky and the Cayston will help keep him healthy enough for long enough that he'll put some weight on over the next 10 days. We have our big end-of-year clinic visit coming up on the 20th and I'd really rather not bring him in with his spine and all his ribs sticking out through his skin like they are now. His appearance doesn't really tell the full story of how hard we have been trying to keep up with nutrition this winter.

For the past couple of months I've been wanting to teach Lemon to swallow pills, since everyone has been getting pretty tired of the whole enzymes with applesauce show. But, with all the puking there hasn't been a good opportunity--the last thing I want is for him to draw a connection between that and swallowing pills. He also hasn't seemed that interested, in spite of not wanting to do the applesauce anymore. But, this evening, he spontaneously mentioned to me that he wants to learn to take pills whole. So, that seems hopeful, and will give us something fun to work on over the holidays. Don't worry, in addition to learning pill swallowing, Grandma and Grandpa are coming to visit and we are going to a water park, so there will be some more traditional fun as well!

Monday, December 4, 2017

Every now and again, maybe about 2 weeks out of every 52 it seems, we get a week where nothing much happens. And man are those the best weeks ever. Lemon and Lime both recovered from the cold they had over Thanksgiving and stayed well for the last 10 days or so. It's amazing what 10 days of good health will do for a couple of boys. By that I mean that their energy level is such that Papa Bear and I have serious concerns as to whether our house and worldly possessions will survive the onslaught. One afternoon this week when Lemon was being particularly "energetic," we at first wondered if something was wrong. It turns out, actually, that something was right--it had been a week or so since he'd last vomited, so he was finally feeling caught up on sleep and nutrition and boy did it show.

I'm glad Lemon was full of pep for his week as "child of the week" in his class at school. He got to wear a crown every day, and have a poster with pictures of his family hung in the classroom. On Wednesday, I went in and did a science demonstration for the kids, layering corn syrup, water, and olive oil in a drinking glass. I think it went well, although thus far Lemon has declined to recount the experience for anyone. After some prodding he is willing to concede that I was in his classroom, but that is about it.

Papa Bear was out of town for a couple of days this week, so at the kids' request I took them on a drive "at night time" (ie 4:45pm) to see the holiday lights around our neighborhood. Since they almost never leave the house when it's dark, this was a profoundly thrilling experience, which we topped off by picking up some take-out food from a restaurant, another cause for high excitement. I couldn't get any traction with Lemon on the chicken satay that I got for him, but weirdly he adored the cucumber salad that came with it. In other news, his relationship with food continues to mystify everyone.

Lime appears to be going through some sort of growth spurt, and I've temporarily conceded the point that he may take a sippy cup of milk to bed with him. The old me would have not been in favor of this idea at all. The new me, on the other hand, recognizes the inherent benefit implied by not getting up to pour a glass of milk at 3 a.m. He's almost up to 25 lb, so who knows, may be by his 30 month check-up in January he will have clawed his way out of the 4th percentile for weight. His little personality is getting more assertive by the day, and he and his brother seem to always want to be together, even when they struggle to maintain domestic harmony. But, being alone is boring. The cats agree!

Monday, November 27, 2017

As if to reaffirm our decision to avoid flying during the winter for the foreseeable future, both kids came down with yet another bad cough on Wednesday. This, and the associated puking (which followed a solid stretch of let's say 3 or 4 puke-free days), made us question the wisdom of continuing with our planned Thanksgiving trip to Milwaukee. But, the prospect of doing nothing except sit around the house and cough for Thanksgiving seemed too grimly depressing, so we packed up everything we own and went ahead with the plan.

Ultimately it was the right choice and I'm glad we did it. I couldn't honestly say that it was a relaxing time, but we had a lot of fun with Nona and Opa and the kids got to experience a lot of new things. We did a lot of swimming in the pool at the hotel, went to a museum and saw some dinosaurs, and went to an aquarium to see some fish. Lime was particularly impressed with all the tall buildings, which is a good reminder that he is really a country mouse, Milwaukee being the biggest city he can ever remember seeing.

The kids were both still pretty sick on Thursday, but by Friday they were starting to turn things around, and today both of them seem nearly recovered. I have to say the one thing I am the most grateful for this year is that I finally feel like we have some degree of control over Lemon's respiratory health. Don't get me wrong, this fall has been pretty much a non-stop series of respiratory viruses of varying intensity that have affected both kids (and their adult companions to varying degrees). But, Lemon has been able to throw off all but one with just increased airway clearance and no antibiotics. It still sucks that he is sick so much, but this is nothing like the bad old days when every time he got a runny nose, Papa Bear and I knew that within 6-8 weeks we would be talking about IV antibiotics. I can't tell you how good it feels to go into a winter and not feel completely and utterly defenseless. It's only November, of course, and there is still a lot of winter to go, but we have fought hard and won a number of rounds already, and any number of wins is more wins than we ever had before.

I wish that I were feeling more bullish on nutrition, our other constant battle ground. Eating is still not really a thing for Lemon. He enjoys food school and will do a lot of things in the room with his therapist, but we haven't seen much translation of those lessons into increased food consumption at home. And, right now we have no room at all to back down on the tube feedings. With all these illnesses and all the puking, we are still at 0 weight gain since June, and that's with us pumping as much formula as we can in through the tube whenever he's well enough to keep it down. I'm starting to feel like maybe the win here is just not losing any further ground until spring, which is kind of a depressing thought but it's good to be realistic I suppose.

In more uplifting news, Lemon is "person of the week" at his school this week so if you'll excuse me, I have to go research science demonstrations that are appropriate for the 4K set and take 15 min to execute...

Monday, November 20, 2017

There is an old Yiddish folk tale about a guy who feels like his house is too crowded and noisy, so he asks his rabbi for advice. The rabbi suggests that the man bring a goat into the house. Predictably, this does not improve matters, so the man goes back to the rabbi. Following a succession of visits to the rabbi, the man has brought basically every animal on his farm into his house. Finally, the rabbi suggests removing all the animals, at which time the man discovers that his home is actually a peaceful paradise.

Our version of this story goes something like, oh, man, having two preschoolers, one of whom has a chronic health condition, is really tough at times. How about if one of the kids randomly starts performing toilet activities not on the toilet? Better? No? How about giving one of those kids the ability to vomit on command? No? That didn't improve things either? How about if we just return to the status quo of two preschoolers, one with a chronic health condition, but no random toilet activities and no vomiting?

You guys, it is paradise on earth over here. I have not washed a puke-soaked thing in several days. What was our genius strategy? We told Lemon that if he kept puking, we could not go on our planned family trip to Milwaukee for Thanksgiving, which involves staying at a hotel with a swimming pool, and possibly also seeing dinosaur fossils at a museum. Like flipping a switch. I can tell that he feels much better, too. After all, throwing up is not pleasant and exhausting, plus which if you retain say zero of the nutrients you are supposed to be ingesting, you feel lousy. Now he is bright eyed and full of energy again. Fingers crossed, we are done with that whole scenario.

The one problem we were still having was with brushing his teeth, where the mere sight of his toothbrush would make him gag violently, and I could only brush about two or three strokes before I had to stop lest risking whatever dinner had made it in coming back out again. I mentioned this problem at food school today and his therapist suggested a new toothbrush. I was skeptical, because I thought it was really the physical sensation of the brush in his mouth that was causing the problem, but given that we are going to the dentist TOMORROW I was willing to try anything. So, after bath this evening, I loaded up the old tooth brush, got so far as about the end of Lemon's nose, and he started gagging. I then pulled a brand new tooth brush out of the drawer, and asked if he wanted to try that one instead. He said yes, so I got it out, and lo and behold, he didn't even flinch, and in fact insisted on taking a turn brushing his own teeth after I was done.

The mind is a powerful force. The young child's mind is both powerful and capricious. Ah well. With a sufficient supply of kittens, running, and bourbon perhaps we'll make it through.

Monday, November 13, 2017

The cough that both kids have had for more than a week is starting to gradually subside. We've been doing three treatments a day for basically two weeks running but (knock on wood) have not felt like we need to start another course of Cayston yet. We are still relying on Lime as our canary in the coal mine--if he's still coughing then the fact that Lemon is coughing too seems less troubling.

I don't usually dwell on this particular topic on the blog, but this cold has brought with it a lot of puking. Even Lime has coughed to that point a few times with this particular bug, and with Lemon every meal and tube feed has been fraught with peril. Ick. Lots of gross laundry, many, many outings with my new steam mop, etc. But, on the plus side, our issue from a few weeks ago of a certain someone performing bodily functions where they should not be performed seems to have stopped of its own accord. So, although there was gross laundry, there was a dearth of other gross messes to be cleaned, which felt like progress.

However. There is always a "however." However, it seems that all this puking from having a cold and a bad cough and swallowing a lot of mucus gave Lemon a new idea. Perhaps he found that being the potty bandit did not result in the amount or caliber of attention that he had in mind. He certainly noticed that if you puke, your parents tend to sort of rush around, act extremely sympathetic, cater to your needs and wants, etc etc. Those of you familiar with kid logic may see where this is going. We had a few puke incidents that came without the usual warning signs, but sometimes there are no warning signs so I didn't think anything of it. Then we had one last night at dinner that seemed really sort of weird, like everything was 100% fine one minute and 100% the opposite of fine the next, where I actually said to Papa Bear afterwards that I suspected Lemon had done it intentionally. But, we both rejected the idea, because really, who does that.

Then, there was this morning at 4 a.m., when Lemon started yelling "Hello!" very cheerfully from his room. He has a stoplight clock, and he knows that he is not supposed to wake us up when the light is red unless he needs help. We'd had a couple of mornings over the past week or two where he's done this "Hello!" routine at ungodly hours and been given a stern reminder about the whole red light concept. So, I stumbled out of bed and over to his room grouchy and annoyed and ready to give the whole red light speech again. But, when I opened his door, he said, with a big grin, "Look, I threw up!" Having no choice, I sprang into action with the whole cleaning routine, while talking to him in a neutral tone about what was going on. Was he feeling sick? No. Was he coughing a lot? No. What happened? He wanted to throw up, so he did. Of course, by this time, all the commotion had woken Lime up, so we all got to start our day, which I suspect was Lemon's objective all along.

I sincerely hope this is a short phase. Or maybe just a one-off. Throwing up isn't fun, right? No amount of getting to sit on top of your grumpy mom and discuss the relative merits of various sports cars at 4:41 a.m. is worth that, is it?

In less gross news, we are still trying to figure out a solution to our overpriced ursodiol problem. We transferred all of our prescriptions from CVS to the children's hospital, and at least there they are only charging us $60 a bottle for the stuff, rather than $90, but $60 is still quite a bit more than the $5 we would like to be paying given that it's a generic medication. We'd like to switch to pills, which we could just crush and give through Lemon's G tube, so I've been in communication with the GI doc's office about that. Well, actually, I have been locked in a MyChart messaging war with a nurse who, how shall I say this, DOES NOT KNOW HOW TO DO MATH. Like, the kind of math you would need to be able to do to divide the number of milligrams in a pill by two and compare the result with the number 170 milligrams to determine if it were a) the same number, b) a smaller number, or c) a larger number, and to make a therapeutic decision on the basis of that comparison. For the moment, I am giving up on this war, because I lack the time and energy. We will just pay for this month and next month's bottles of the compounded stuff, and then see how Lemon's liver enzymes look in December. If the ursodiol seems to be doing something (in which case we will continue it long-term), perhaps I will be able to find a way to actually speak to the doctor, who presumably understands both math and the concept of therapeutic range, and we can work out an actual solution.

A closing note from the "height of irony" department. Lemon's Pulmozyme prescription is filled by CVS specialty pharmacy. Theoretically, it is possible to get the prescription set up to auto-refill. I say theoretically, because Papa Bear is among the most intelligent and diligent of pharmacy customers, and for over a year he has been unable to achieve this despite innumerable attempts. Finally, today, he succeeded. And, tomorrow, he will have to call and transfer the prescription to our new pharmacy. Victory!

Monday, November 6, 2017

We have certainly come a long way from where we were a year ago. Halloween reminded us that at this time last year, we were doing home IVs, and Lemon in fact had to go trick-or-treating wearing a backpack containing his IV pump. This year, I kept alive the "trick" in trick-or-treat by bringing the kids to get their annual flu shots on Halloween morning. Both kids were very dramatic about the whole thing, wailing about the indignity and injustice of it until I said, "Hey, that lady is dressed in a pilot uniform. And she is offering you stickers." End tears, return to school.

We enjoyed a week of relative good health here before a new cold hit the dynamic duo on Friday into Saturday. Now we're back to having our house sound like some kind of sanatorium, and poor Lime has coughed himself so hoarse that he sounds like a little frog. Lemon's voice is unaffected but he too has been coughing up a storm, with all the attendant setbacks in terms of nutrition. At this point I feel like if we just hold our ground we'll be winning--the possibility of exceeding his June weight any time soon seems remote. The good news is that in spite of having this pretty bad virus both kids are in good spirits, enjoying the kittens, and generally having a good time. The bad news is that we've had to miss food school again, the third time in 6 weeks. I am really wondering whether it's even worth continuing to pursue it in the winter, or whether we'd be better served by just waiting for spring again. Winter is just not an eating season for Lemon.

We got an upsetting letter in the mail this week: as of January 1, our insurance will no longer cover the pharmacy that we have been using since we moved here (formerly Target, now CVS within Target). When you fill as many prescriptions per month as we do (see illustrative photo), the relationship with the pharmacy is an important one, and we'd invested 4 years in this one, figuring out who the good and bad pharmacists were, getting the "auto" refill system to work the way we wanted, negotiating the copay cards and specialty pharmacy and all the rest. We're switching to the pharmacy at our children's hospital, on the theory that perhaps a pharmacy affiliated with a hospital might be able to handle a complex prescription load. Well, that and the fact that they aren't Walgreens, the other choice now offered by our insurance.

Another loss this week: the cart that we've used for Lemon's vest and associated equipment since we started on the vest reached the end of its service life this week. The kids and I had a good time putting together its replacement (thank you, Amazon, for remembering exactly what we ordered last time). It's hard to believe that we are already two years into using the vest. I recently ordered Lemon the next size up on the vest itself, since he'd outgrown the old one. Being aware of his deeply conservative nature, I ordered him the exact same color, just in a bigger size. I thought he'd be excited when the new one came, but instead he refused to even try it on. He was only willing to try it on when I promised that it was just a test, and that he would still use the old one. He did not anticipate that I would be so sneaky as to swap the old one out for the indistinguishable new one when he wasn't looking. The depths that parenting pushes you to...